| Literature DB >> 21997512 |
Guillaume Ssi-Yan-Kai1, Julie Vial, David Labarre, Stéphane Decramer, Christiane Baunin.
Abstract
We report a rare example of anaemia and hypertension due to an incomplete Carney triad in a 14-year-old girl with no previous medical history. This rare non-familial syndrome generally involves two disparate tumours: gastrointestinal stromal tumour, paraganglioma and/or pulmonary chondroma. The complete triad is a syndrome that involves at least five loci: stomach, oeophagus, lung, the paraganglionic system, adrenal (cortex or medulla). The pathogenesis is unclear as these tumours have different embryological origins. Surgical treatment is necessary, and long-term follow-up is advisable as patients with Carney triad may re-present with tumour(s), even several years after the first presentation.Entities:
Mesh:
Year: 2011 PMID: 21997512 DOI: 10.1007/s00247-011-2241-x
Source DB: PubMed Journal: Pediatr Radiol ISSN: 0301-0449